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Diagnosing Dysarthria in Adults. A New Speech Assessment Method for Polish, English, and Spanish

D I AGNOS I NG DYSARTHR I A I N ADULTS

BEYOND LANGUAGE The series under the auspices of: College for Interdisciplinary Studies, University of Wrocław, Poland Kolegium Międzyobszarowych Studiów Indywidualnych, UWr In cooperation with: College for International Interdisciplinary Studies in Humanities and Social Sciences, Adam Mickiewicz University in Poznań, Poland Międzyobszarowe Indywidualne Studia Humanistyczne i Społeczne UAM and Faculty of History, Adam Mickiewicz University in Poznań Wydział Historii, Uniwersytet im. Adama Mickiewicza w Poznaniu Committee for Philology of the Polish Academy of Sciences, Wrocław Branch Komisja Nauk Filologicznych Oddziału PAN we Wrocławiu Scientific Board Committee for Philology of the Polish Academy of Sciences, Wrocław Branch Andrei Avram (Bucharest, Romania) | Jerzy Axer (Warsaw, Poland) | Katarzyna Buczek (Opole, Poland) | Piotr Cap (Łódź, Poland) | Lorenzo Calvelli (Venice, Italy) | Tadeusz Cegielski (Warsaw, Poland) | Piotr P. Chruszczewski (Wrocław, Poland) | Camelia M. Cmeciu (Bucharest, Romania) | Marta Degani (Verona, Italy) | Michel DeGraff (Boston, USA) | Robin Dunbar (Oxford, UK) | Katarzyna Dziubalska-Kołaczyk (Poznań, Poland) | Joanna Esquibel (San Diego, USA) | Ray Fabri (La Valetta, Malta) | Franck Floricic (Paris, France) | | Stanisław Gajda (Opole, Poland) | Piotr Gąsiorowski (Poznań, Poland) | Yeshaya Gruber (Jerusalem, Israel) | Franciszek Grucza (Warsaw, Poland) | Kazimierz Ilski (Poznań, Poland) | Rafael Jiménez Cataño (Rome, Italy) | Ewa Kębłowska-Ławniczak (Wrocław, Poland) | Grzegorz A. Kleparski (Rzeszów, Poland) | Konrad Klimkowski (Lublin, Poland) | Aleksandra R. Knapik (Wrocław, Poland) | Tomasz P. Krzeszowski (Warszawa, Poland) | Marcin Kudła (Rzeszów, Poland) | Christopher Laferl (Salzburg, Austria) | Barbara Lewandowska-Tomaszczyk (Łódź, Poland) | Marcin Majewski (Kraków, Poland) | Rafał Molencki (Sosnowiec, Poland) | Marek Paryż (Warsaw, Poland) | John Rickford (Stanford, USA) | Hans Sauer (Munich, Germany) | Waldemar Skrzypczak (Toruń, Poland) | Agnieszka Stępkowska (Warsaw, Poland) | Aleksander Szwedek (Poznań, Poland) | Elżbieta Tabakowska (Kraków, Poland) | Jerzy Wełna (Warsaw, Poland) | Donald Winford (Columbus, USA) | Anna Wojtyś (Warsaw, Poland) | Przemysław Żywiczyński (Toruń, Poland)

I ZABE LA GATKOWSKA D I AGNOS I NG DYSARTHR I A I N ADULTS , 2020 A NEW SPEECH ASSESSMENT METHOD FOR POL ISH, ENGL ISH, AND SPANISH

Diagnosing Dysarthria in Adults. A New Speech Assessment Method for Polish, English, and Spanish Title of the Series: Beyond Language, Vol. 3 Text © 2020 Izabela Gatkowska Copyright for this edition © 2020 Æ Academic Publishing All rights reserved. Except as permitted under the U.S. Copyright Act of 1976, no part of this publication may be reproduced, distributed, or transmitted in any form or by any means, or stored in a database or retrieval system, without the prior written permission of the publisher. Editors-in-Chief: Prof. Piotr P. Chruszczewski (Wrocław) Dr. Aleksandra R. Knapik (Wrocław) Editors for the Series: Dr. Katarzyna Buczek (Opole) Dr. Marcin Kudła (Rzeszów) MISHiS UAM Prof. Aleksander W. Mikołajczak (Poznań) Co-Editors: Prof. Konrad Dominas (Poznań) Prof. Rafał Dymczyk (Poznań) Honorary Editors Prof. Michel DeGraff (Boston, MA) for the Series: Prof. Isaiah Gruber (Jerusalem) Prof. Christopher F. Laferl (Salzburg) Æ Managing Editor: Dr. Joanna Esquibel Translator: Daniel Sax Line Editor: Nicholas Z. GodBossel Phonology Reviewer: Dr. Marta Domagała The Author wishes to thank Dr. Javier Arias Navarro for the Spanish language consultation and Nigel Gotteri, Honorary Senior Lecturer in Russian and Slavonic Studies, University of Sheffield, UK, for providing English subtitles for the patient recordings. Publication subsidized by the Jagiellonian University in Kraków, Poland, from the funds of the Faculty of Management and Social Communication. Æ Academic Publishing 501 W. Broadway Ste A186 San Diego, CA 92101, USA www.aeAcademicPublishing.com contact@aeAcademicPublishing.com 1st international edition: Æ Academic Publishing, 2020 Library of Congress Control Number: 2020943553 ISSN: 2642-6951 (print), 2642-696X (online) ISBNs: 978-1-68346-181-4 (pbk) 978-1-68346-182-1 (mobi) | 978-1-68346-183-8 (ePub) 24 23 22 21 20 1 2 3

TABLE OF CONTENTS List of Recordings .......................................................................................... vii List of Abbreviations ..................................................................................... ix List of Selected IPA Symbols . ........................................................................ x INTRODUCTION ....................................................................................... 1 1. Physiological Foundations of Speech (Material to Dysarthria) ........... 3 1.1. Physiological foundations of speech .................................................... 3 1.2. Vocalization and production of speech sounds . ................................... 4 1.3. Prosodic elements .............................................................................. 6 1.3.1. Phoneme length ...................................................................... 6 1.3.2. Word and sentence stress ......................................................... 7 1.3.3. Intonation patterns . ................................................................ 9 1.4. The role of the brain and cerebellum in executive aspects of speech production ......................................................................................... 11 2. Dysarthria: Changes in the Clinical Picture of Speech ........................ 13 2.1. Phonetic distortions ........................................................................... 16 2.2. Prosodic distortions ............................................................................ 24 2.2.1. Phonation disorders ................................................................. 25 2.2.2. Intonation and stress disorders ................................................. 26 2.2.3. Fluency disorders ..................................................................... 27 2.3. Dynamics of change in the clinical picture of speech . ......................... 28 3. Dysarthria vs. Other Organic Speech Disorders in Adults .................. 30 3.1. Dysarthria vs. aphasia ........................................................................ 30 3.2. Dysarthria vs. tongue apraxia . ........................................................... 32 3.3. Dysarthria vs. anarthria ..................................................................... 32 3.4. Dysarthria vs. dementia ..................................................................... 32 4. Diagnosing Dysarthria ........................................................................... 34 4.1. A New Speech Assessment Method of dysarthria diagnosis: diagnostic process ............................................................................................... 36 4.1.1. Articulation ............................................................................. 36 4.1.2. Consonant and vowel length . .................................................. 39 4.1.3. Intonation ............................................................................... 40 4.2. Patient interview . .............................................................................. 42 4.2.1. Fine motor control ................................................................... 43

vi Diagnosing Dysarthria in Adults 4.2.2. Breathing and phonation ......................................................... 44 4.2.3. Nasality . ................................................................................. 46 4.2.4. Consonant and vowel length . .................................................. 46 4.2.5. Reading aloud ......................................................................... 46 4.2.6. Question asking . ..................................................................... 47 4.2.7. Sentence repetition .................................................................. 47 4.2.8. Handwriting . .......................................................................... 47 4.2.9. Additional tests ....................................................................... 48 5. Dysarthria In Various Neurological Diseases ........................................ 50 5.1. Dysarthria accompanying motor neuron diseases (MNDs) . ................ 51 5.1.1. Flaccid dysarthria in MND ...................................................... 53 5.1.2. Spastic dysarthria in MND ...................................................... 56 5.1.3. Mixed flaccid–spastic dysarthria in MND ................................ 58 5.2. Dysarthria accompanying Parkinson disease (PD) .............................. 61 5.2.1. Hyperkinetic dysarthria with hypokinetic elements in PD ....... 62 5.2.2. Hypokinetic dysarthria in PD .................................................. 64 5.2.3. Severe hypokinetic–hyperkinetic dysarthria in PD ................... 67 5.3. Dysarthria accompanying Parkinsonism ............................................. 68 5.3.1. Example of hypokinetic dysarthria in Parkinsonism . ................ 68 5.4. Dysarthria accompanying Huntington disease (HD) .......................... 69 5.4.1. Hyperkinetic (dyskinetic) dysarthria in HD in older age .......... 71 5.4.2. Dysarthria in the juvenile form of HD ..................................... 73 5.5. Dysarthria accompanying laryngeal dystonia: various dysarthric forms depending on etiology .............................................................. 76 5.6. Dysarthria accompanying multiple sclerosis ....................................... 80 5.6.1. Example of ataxic–spastic dysarthria in MS .............................. 81 5.7. Dysarthria accompanying spinocerebellar ataxia . ............................... 83 5.8. Dysarthria accompanying progressive supranuclear palsy ................... 86 5.9. Dysarthria accompanying multiple-system conditions ........................ 88 5.9.1. Ataxic–spastic–hypokinetic dysarthria in disorder of pyramidal, extrapyramidal and cerebellar systems ................ 88 5.9.2. Ataxic–spastic dysarthria in multiple-system atrophy (MSA) . .. 90 5.10. Summary of diagnostic cases . ............................................................ 91 CONCLUSIONS . ......................................................................................... 94 Appendix ...................................................................................................... 95 Speech Assessment Method (SAM) ................................................................ 97 References ..................................................................................................... 104 Further reading ............................................................................................. 107 Index . ........................................................................................................... 109

LIST OF RECORDINGS  Recording 1. [67 yr old male] Flaccid dysarthria in MND .............................................................. 54  Recording 2. [57 yr old female] Spastic dysarthria in MND............................................................... 56  Recording 3. [64 yr old female] Mixed flaccid–spastic dysarthria in MND......................................... 58  Recording 4. [75 yr old male] Hyperkinetic dysarthria with hypokinetic elements in PD................ 62  Case 1. Recording 5. [58 yr old female] Hypokinetic dysarthria in PD. ......................................................... 64  Case 2. Recording 6. [35 yr old male] Hypokinetic dysarthria in PD. ......................................................... 65  Recording 7. [62 yr old male] Severe hypokinetic–hyperkinetic dysarthria in PD............................ 67  Recording 8. [42 yr old female] Hypokinetic dysarthria in Parkinsonism........................................... 68  Case 1. Recording 9. [58 yr old male] Hyperkinetic (dyskinetic) dysarthria in HD (older age)..................... 71  Case 2. Recording 10. [56 yr old female] Hyperkinetic (dyskinetic) dysarthria in HD (older age)..................... 73  Case 1. Recording 11. [27 yr old male] Dysarthria in HD (juvenile form)..................................................... 73  Case 2. Recording 12. [20 yr old male] Dysarthria in HD (juvenile form)..................................................... 74  Case 1. Recording 13. [62 yr old male] Dysarthria in laryngeal dystonia....................................................... 77  Case 2. Recording 14. [52 yr old female] Dysarthria in laryngeal dystonia....................................................... 78  Recording 15. [34 yr old male] Ataxic–spastic dysarthria in MS........................................................ 82  Recording 16. [57 yr old female] Ataxic dysarthria in spinocerebellar ataxia........................................ 84  Recording 17. [57 yr old male] Spastic dysarthria in PSP.................................................................. 86

viii Diagnosing Dysarthria in Adults  Recording 18. [66 yr old female] Ataxic–spastic–hypokinetic dysarthria in pyramidal, extrapyramidal, and cerebellar system disorder.......................................................... 88  Recording 19. [61 yr old female] Ataxic–spastic dysarthria in MSA..................................................... 90 Information on all Recordings available at Æ Academic’s page: https://aeacademicpublishing.com/books/diagnosing-dysarthria/

LIST OF ABBREVIATIONS ASSIDS – Assessment of Intelligibility of Dysarthric Speech ALS – a myotrophic lateral sclerosis (cf. MND), also: Charcot disease,1 Lou Gehrig disease CT – computer tomography EMG – Electromyography ET – essential tremor FDA – Frenchay Dysarthria Assessment fMRI – functional magnetic resonance imaging HD – Huntington disease LD – laryngeal dystonia MND – motor neuron disease MRI – magnetic resonance imaging MS – multiple sclerosis, also: disseminated sclerosis MSA – multiple-system atrophy ORL – otorhinolaryngology, also: otolaryngology PD – Parkinson disease POS – psychoorganic syndrome PSP – progressive supranuclear palsy RDP – Robertson Dysarthria Profile SAM – Speech Assessment Method SCA – spinocerebellar ataxia SN – substantia nigra TBI – traumatic brain injury 1 [Note: The BL series follows WHO and AMA guidelines, which advocate for the elimination of the possessive in eponymic names of neurological disorders. For details, see, e.g. Michael R. MacAskill, Tim J. Anderson (2013) “Whose name is it anyway? Varying patterns of possessive usage in eponymous neurodegenerative diseases.” PeerJ 1(e67); DOI: 10.7717/peerj.67—ed.’s note]

LIST OF SELECTED IPA SYMBOLS æ open front unrounded oral vowel β voiced bilabial fricative ɕ voiceless (alveolo)palatal fricative ð voiced dental fricative d͡ʒ voiced palatoalveolar affricate ə mid central unrounded oral vowel (schwa) ɚ rhotacized mid central unrounded oral vowel ɛ open-mid front unrounded oral vowel ɛ̃ open-mid front unrounded nasalized vowel h voiceless glottal fricative x voiceless velar fricative ɪ lax close front unrounded oral vowel ɨ high central unrounded oral vowel j oral palatal approximant l voiced alveolar lateral approximant ɫ velarized voiced alveolar lateral approximant ɱ voiced labiodental nasal stop ŋ voiced velar nasal stop ɲ voiced palatal nasal stop ɔ open-mid back rounded oral vowel ɔ̃ open-mid back rounded nasalized vowel ɾ voiced alveolar tap ɹ voiced postalveolar approximant r voiced alveolar trill ʃ voiceless palatoalveolar fricative ʂ voiceless postalveolar fricative θ voiceless dental fricative t͡ʃ voiceless palatoalveolar affricate t͡s voiceless alveolar affricate ʊ lax close back rounded oral vowel ʌ open-mid back unrounded oral vowel ɣ voiced velar fricative ʒ voiced palatoalveolar fricative ʐ voiced retroflex fricative ʑ voiced alveolopalatal fricative ⟨⟩ spelling // phonemic transcription : long vowel ‘ primary stress

INTRODUCTION D y s a r t h r i a is the name of a class of organic speech sound disorders which impair the executive aspects of human speech, i.e. the function of the articulatory organs, the movements of the tongue, lips, and soft palate. The impairment of specific elements of the speech apparatus gives rise to qualitative changes in the speech sounds, possibly in tandem with shortened phonation duration and prosodic disorders, mainly affecting intonation and word stress. The speaker’s voice quality and pace of speech are also altered. This set of dysarthric symptoms in speech may appear secondary to a neurological disease, irrespective of the specific language spoken by the patient. Changes in the clinical picture of speech may sometimes become evident before the full manifestation of the neurological condition, therefore contributing to an earlier diagnosis. When speech changes are audible, professional speech analysis yielding a good and accurate diagnosis of dysarthria may be indicative of a particular neurological disease and thus confirm a neurological diagnosis, or – in the case of an unclear presentation – it may help resolve medical doubts. From the physiological perspective, human speech organs do not differ substantially, irrespective of a language spoken. Hence, when a dysarthria-causing disease impairs the function of the articulatory organs (mainly the tongue but also the lips and soft palate) and causes the phonation duration to be shortened, the pace of speech to be altered, and qualitative changes to arise in the patient’s voice, all these changes occur independently of the patient’s native language. We should interpret impaired prosodic elements similarly (mainly utterance intonation and word stress), as these changes are also independent of the language. Since the human speech apparatus is physiologically roughly the same in speakers of all languages, we may assume that its defects caused by dysarthria will manifest in speech in the same way. Granted, the phonetic systems (systems of speech sounds) of the Indo-European languages differ in terms of their inventory of sounds, sound length, prosody, accentual system, etc. However, speech apparatus defects will most likely manifest in the same speech sound groups produced by identical or similar articulatory processes across languages. The problem that the diagnostician faces, therefore, involves identifying those sounds in various languages and linking them to a specific disorder.

2 Diagnosing Dysarthria in Adults The point of departure for the current study is an analysis of the speech impairments observed in adult neurological patients speaking Polish. First, I analyzed the acoustic (phonetic) defects in order to identify the articulatory processes impaired by a neurological disorder. In the next step, I identified the group of speech sounds that are produced by these same articulatory processes in English and Spanish. This shall allow the diagnostician to focus observation on these specific groups of sounds, since their distortion may indicate impairment of the articulatory apparatus. This original, linguistically-grounded method of diagnosing dysarthric speech disorders, developed and tested in a neurological clinic for Polish-speaking patients, was adapted for the diagnostic needs of English- and Spanish-speaking patients (Gatkowska 2012). The new s p e e c h a s s e s s m e n t m e t h o d (SAM) tests the efficacy of the tongue, lips, and soft palate in speech production by incorporating selected sounds present in the phonetic system of a given language. Other diagnostic tasks to test articulatory organ function can be used irrespective of the patient’s language. Diagnostic tests to evaluate the intelligibility of speech, prosodic elements, phonation, and writing ability (distinctively misshapen letters are an indication for neurological diagnosis) have been adapted for individual languages, i.e. Polish, English, and Spanish. In developing the new SAM, I followed what may be described as a certain “happy medium” methodology, striving to strike an ideal balance between minimizing the number of diagnostic tasks required of the patient and maximizing the informational value of the tasks, in order to facilitate an accurate diagnosis of dysarthria. The discussion of SAM is illustrated with examples of its practical application. The attachment contains 19 recordings that illustrate diagnostic testing of various forms of dysarthria carried out at the Neurology Clinical Department, Collegium Medicum, Jagiellonian University in Kraków, Poland, between 2010 and 2011. The patients are speaking Polish, and so their recordings are subtitled in English. Various forms of dysarthria recorded in the films are described and interpreted in Chapter Five. A full list of diagnostic tasks and instructions given by the diagnostician is provided in the Appendix. Izabela Gatkowska Kraków, May 2019

1. PHYSIOLOGICAL FOUNDATIONS OF SPEECH (MATERIAL TO DYSARTHRIA) 1.1. PHYSIOLOGICAL FOUNDATIONS OF SPEECH At the very start, I should draw a clear distinction between the terms: “language” and “speech.” A l a n g u a g e is defined as a system of symbols (linguistic signs) and a set of combinatory rules for creating sentences out of those symbols. Each symbol, i.e. each word or phrase, has both form and meaning, and can therefore be used for conveying information. S p e e c h , on the other hand, refers to a specific act or acts of language use for the sake of conveying information. For a speech act to be received by an addressee, the form of a symbol has to be physically realized, i.e. as sound or as a graphic representation. Before a person speaks (performs a speech act), they entertain a thought, which, as a pre-verbal representation, is difficult to investigate. What can be studied is the verbal counterpart of a conceptual representation that appears at the verbalization stage. This stage starts with u t t e r a n c e p l a n n i n g , i.e. selecting words, constructing the sentence, adjusting the grammatical forms to syntactic requirements, which is then followed by u t t e r a n c e e xe c u t i o n , i.e. physically uttering the sentence. From the neurophysiological perspective, the execution phase involves finding the right kinesthetic engrams to pronounce single phones/speech sounds and combinations thereof, and then constructing grammatical forms of words and larger complexes, such as phrases, sentences, or texts. This process can be represented as follows: THOUGHT → UTTERANCE PLANNING → EXECUTION It is assumed that the different levels of language representation correspond to different types of brain activity necessary for their materialization in communicative acts. The phase of planning an utterance requires neural processes known as s p e e c h mo t o r p r o g r a mm i n g m e c h a n i s m s . The production phase is effectuated by s p e e c h e x e c u t i o n m e c h a n i s m s ,

4 Diagnosing Dysarthria in Adults responsible for phonation, articulation, and prosody, including intonation, rhythm, and speech rate (tempo). Speech planning has to be seen as distinct from execution, and this distinction is crucial in accounting for speech disorders of the dysarthria type. Dysarthria is defined as a disorder of speech production resulting from organic causes. Speech planning, i.e. selecting words or constructing sentences, is therefore not affected in dysarthria; nor are language comprehension, vocabulary resources, or the functioning of the semantic memory (unless dysarthria is accompanied by other disorders, such as aphasia or dementia, which can cause additional problems with comprehension, attention, speech planning, and production). Rather, the pathological symptoms characteristic of dysarthria manifest themselves in speech motor production, specifically including impaired articulation, problems with producing nasal/oral features of speech sounds, problems with rendering prosodic features of an utterance, such as intonation, stress, speech rate (which can become accelerated, decelerated or varied), shortened phonation time (i.e. voicing production), possible breathing disorders (such as shortened breath or loud inspiration/expiration), or various voice disorders, such as diminished intensity or qualitative disorders, including hoarseness, lack of sonority, flat sound, etc. Before I discuss the dysarthria-related pathological changes in speech production in detail, however, let us first review the most essential aspects of speech production in general. 1.2. VOCALIZATION AND PRODUCTION OF SPEECH SOUNDS Speech production involves five independent physiological processes, i.e. articulation, breathing, phonation, resonance, and prosody. The physiological actions underlying speech production therefore involve collaboration among a number of different organs. Articulation engages the tongue, lips, lower jaw, palate, larynx, and respiratory muscles. Phonation also usually requires an appropriate airflow, proceeding from the lungs, bronchial tubes, and trachea, to the larynx, which amplifies the voice’s fundamental frequency, and the pharynx, nasal and oral cavities—all responsible for the timbre of the voice and for producing specific speech sounds (Minczakiewicz 1990, Ichikawa & Kageyama 1991). Breathing itself is the work of the lungs, thoracic cage, diaphragm, and abdominal muscles. For breathing to take place, central control has to be exercised over the respiratory muscles. The respiratory system carries the

5 1. Physiological Foundations of Speech (Material to Dysarthria) airstream to the subglottal location in the larynx in order to produce an air column necessary for the correct functioning of the larynx and vocal folds (Shprintzen & Siegel-Sadewitz 1982; Minczakiewicz 1990). This is followed by phonation, in which the larynx plays the main role. Variations in phonation occur depending on the length and thickness of the vocal folds, as well as their position and vibrations during vocalization. The frequency of vocal fold vibration is unique for each person, corresponding to the pitch of the voice produced by the larynx (Ichikawa & Kageyama 1991). Another essential parameter of speech is the nasal/oral quality of a speech sound. The function of resonator is fulfilled by the vocal channel consisting of the oral cavity and pharynx (oropharynx), together with the resonating space of the lateral nasal cavities (nasopharynx) connected to the respiratory channel. The supraglottal space forms a cavity in which fundamental frequencies resonate as harmonic variants. The phoneme, in general, is a class of phonetically related speech sounds which do not co-occur in the same environment (see Wróbel, Dukiewicz & Sawicka 1995); to make the definition less abstract, cf. the explanation from Lyons: In English, p, t and k in certain positions of the word are slightly aspirated (that is, pronounced with an accompanying slight puff of breath); in other positions, after s for example, they are unaspired (cf. top : stop, pot : spot, etc.). In a broad transcription, therefore, the phonetician might well use the same letter (or other symbol) to represent both the English speech-sound (as indeed the alphabet used for English generally does), although they are quite easy distinguishable, phonetically. […] The reason why we can present both the aspired and the unaspired consonants with the same symbol in broad transcription is that the distinction between the aspired and the unaspired variety never has the function of keeping apart different words in English (this is a very crude, and partly inaccurate, statement of a principle that will be treated more fully later); it is not a functional difference: it is a phonetic difference, but not a phonological, or phonemic, difference in English […]. In cases of this kind we say that the phonetically distinguishable […] speechsounds are positional variants, or allophones, of the same phoneme. They are called positional variants because the occurrence of one rather than another of phonetic variants of a particular phoneme is determined by the position of the phoneme in the word. (Lyons 1968: 100–101) Phonemes fall into distinctive types, depending on the parts of the speech organs engaged (tongue, lips, cheeks, and soft palate): e.g. the nasopharynx

6 Diagnosing Dysarthria in Adults acts as a nasal resonator for the consonants m, n,1 whereas the closure of the palate yields consonants such as b, p, k (Shprintzen & Siegel-Sadewitz 1982; Minczakiewicz 1990; Kent et al. 1992). The quality of articulation is affected by the position and condition of the articulatory organs and by the opening of the vocal channel, which is required to be opened for vowels, closed or narrowed for obstruent consonants, and partially opened for sonorant consonants (e.g. m, l). Voiceless stops (k, p) are articulated with the voice channel closed at various positions. 1.3. PROSODIC ELEMENTS This section briefly discusses phoneme length, rhythm, melody, stress, and intonation, which are all features of speech invariably affected by dysarthria. Before discussing pathological changes manifested in various dysarthria forms, a general characterization of prosodic elements will be provided, illustrated with examples from Polish, English, and Spanish. 1.3.1. Phoneme length In some languages, different durations of articulation of certain speech sounds – vowels or consonants – may contribute to differences in meaning. In contemporary Polish, phoneme is not distinctive in this sense, therefore it is not relevant perceptually, either. As far as English is concerned, vowel length and vowel combinations are of crucial importance. Despite this, for the purposes of dysarthria diagnostics, not all vowel length distinctions are diagnostically significant. We need to remember not to burden a patient excessively with diagnostic tasks; a limited number of examples is sufficient to test for pathological patterns in vowel length. For example, if a patient is found not to be able to articulate /ʊ/ and /u:/ distinctively, as in pull /pʊl/ and pool /pu:l/ (BrE), this is symptomatic enough of a problem with vowel duration. In Spanish, on the other hand, we may test for a phonemic difference in the realization of the consonant r in pairs such as, e.g. pero ‘but’ vs. perro ‘dog.’ 1 To make the text more accessible to non-linguists, in the running text alphabetic transcription is applied instead of the phonetic one, unless necessary and then specifically indicated otherwise.

7 1. Physiological Foundations of Speech (Material to Dysarthria) Taking the above into account, speech sound length is included in the battery of tests developed for the Spanish and English version of the diagnostic method proposed here, since it is certain to manifest itself in the prosodic sphere of these languages. In the Polish version this phenomenon is disregarded as perceptually irrelevant. 1.3.2. Word and sentence stress Word stress involves giving acoustic prominence to at least one syllable in a word. Sentence stress, in turn, is logically motivated and involves augmented vocal prominence of a word which carries special affective or conceptual significance. From the perspective of dysarthria diagnostics, sentence stress is more important than word stress. The freely spoken form of Polish, which is considered in the diagnosis of dysarthria, is not characterized by a strong rhythm. Some rhythmic patterns are nevertheless perceived, and so are departures from such regular patterns. In Polish, in contradistinction to Spanish for instance, word stress is fixed in the sense that it typically falls on the penultimate syllable (paroxytone), which sometimes requires shifts from one morphological unit to another in response to morphological suffixation (e.g. stary2 ‘old,’ singular masculine nominative, vs. starego3 ‘old’ singular masculine genitive). In certain words, the stress exceptionally falls on the antepenultimate syllable (proparoxytone)—these include verbs in the 1st and 2nd person plural of the past tense, in all persons singular, and in the 3rd person plural of the conditional mood, numerals from 400 to 900, as well as in foreign borrowings that have not been fully assimilated to the Polish stress pattern, such as (fizyka4 ‘physics,’ matematyka5 ‘math,’ uniwersytet6 ‘university’). There are also some words in Polish with the stress falling on the preantepenultimate syllable, such as verbs in the 1st and 2nd person plural in the conditional mood, e.g. rozmawialibyśmy7 ‘we would (have) talk(ed).’ 2 [For ease of use, here, and elsewhere, boldface indicates the stressed syllable.—ed.’s note]. IPA transcription: /ˈstarɨ/. 3 IPA transcription: /staˈrɛgɔ/ 4 IPA transcription: /ˈfizɨka/ 5 IPA transcription: /matɛˈmatɨka/ 6 IPA transcription: /uɲiˈvɛrsɨtɛt/ 7 IPA transcription: /rɔzmaˈvʲalibɨɕmɨ/

8 Diagnosing Dysarthria in Adults In English, a stress-timed language, correct application of word stress is of particular importance, as it is phonemic, i.e. word meaning can change with a shift in word stress, e.g. upset (the verb /ʌpˈset/ vs. the noun /ˈʌpset/). It has to be noted, though, that some words can have two correct stress patterns. Function words, such as auxiliary verbs, pronouns, or prepositions, also bear different or no stress, depending on their position and function. At the phrase level, stressed syllables carrying a rhythmic accent are considered to also carry a pitch accent if they begin a tone unit (except for low-pitch intonation at the beginning of a phrase). A pitch accent usually falls on the initial syllable of the final rhythmic unit in the fall-rise contour. Other than that, rhythmically accented syllables do not have any intonation prominence. English differentiates between syllables in four different prosodic situations: (1) unstressed syllables; (2) stressed syllables given rhythmic prominence; (3) stressed syllables given rhythmic and intonation prominence (pitch accent); and, finally, (4) nuclear stressed syllables, which are prominent rhythmically and intonationally. The latter tone units are also usually the most salient, meaning-wise. Should any preceding segments within a phrase be given a stronger pitch accent, it only adds more markedness to the melody and meaning of the utterance. Accentuation at the phrase-level, then, may also be of intonational significance. The most salient tone unit, carrying the maximum information load and receiving the pitch accent, is referred to as the nucleus. The only obligatory element of any utterance, the nucleus is also usually its final element, and sometimes the only one. Cf. e.g., the imperative: (1) Come! Stressed syllables are found in any other tone units comprising the head and body of an utterance, before the nucleus, while unstressed syllables comprise the utterance’s pre-head and tail. Cf.: (2) I’d rather have a cup of tea. When the penultimate tone unit has a fall-high contour and the ultimate is low-rise, they both constitute a nucleus, cf.: (3) I like tea. Since dysarthric changes in the melody of speech are of particular interest here, it is always worth evaluating the patient’s speech at the utterance level, to assess whether intonation patterns remain natural or if they have become

9 1. Physiological Foundations of Speech (Material to Dysarthria) distorted (for discussion on prominence, accent, and rhythm cf. also Cruttenden ([1962] 2014: 270–304). In Spanish there are two main principles governing stress, i.e., (1) if a noun ends with a vowel (a, o, e, i) or with the consonant s or n, then the stress falls on the penultimate syllable, e.g. casa ‘house,’ padre ‘father’; (2) if a singular noun ends with a consonant other than those mentioned above (r, t, l, d, etc.), then the stress usually falls on the ultimate syllable, e.g. universidad ‘university,’ feliz ‘happy,’ estar ‘to be.’ Spanish adjectives, adverbs, and verbs in present tense mostly follow the same principle; in other tenses, verb stress may also be governed by morphological principles. Another form of stress attested in Spanish is the graphic accent (acento gráfico), marking a departure from the above principles. Here, the graphic stress appears on the vowel in the strongest syllable, as in está ‘is’ or fácil ‘easy.’ The graphic stress is also used to distinguish between two one-syllable words, e.g. aun ‘even’ vs. aún ‘still/yet’ or te ‘you’ vs. té ‘tea,’ etc. Interrogative pronouns are obligatorily marked with the graphic stress, e.g.: (4) ¿Qué quieres? Apart from that, the graphic stress appears in all words in which the antepenultimate syllable is stressed, e.g. sábado ‘Saturday.’ The same principle is followed in complex forms of verbs, e.g. dando vs. dán-do-lo (inflected and compounded forms of dar ‘to give’). The graphic stress is also marked over the stressed vowels i and u, when they neighbor the vowels a, e, o. In such cases i and u do not form diphthongs but stand on their own as separate syllables, e.g. pa-ís /paˈis/ ‘country’ (if there is a mute ⟨h⟩, it is disregarded). Since the stress falls on the same syllable in the singular and in the plural, when the latter is created for nouns and adjectives by adding the ending -es, the word gains one more syllable, which may in turn necessitate adding the graphic stress or removing it from the plural number. Typically, this kind of situation occurs in words ending with n or s, with the graphic stress falling on the ultimate syllable (e.g. inglés ‘English’), cf. e.g. Lang (1990). 1.3.3. Intonation patterns Intonation involves modulating one’s voice by lowering or raising the pitch, which adds a melodic quality to an utterance. In all three languages considered herein, i.e. Polish, English, and Spanish, intonation carries information about the speaker’s intention concerning the speech act type (interrogative or declarative) and helps in delineating phrases and utterances. Generally,

10 Diagnosing Dysarthria in Adults intonation patterns can be rising or falling, although these are operationalized differently in each of the three languages. Intonation in Polish is founded upon logical and emotional factors, its main aim being to express the speaker’s attitude toward the content uttered. An utterance with a falling intonation pattern (cadence) starts with a higher pitch and ends with a lower pitch. In an utterance with rising intonation (anti-cadence) the reverse is the case. When the speaker’s attitude is objective, the intonation patterns is typically rise–fall. A subjective attitude, on the other hand, is expressed by means of the rising intonation in questions and the falling intonation in exclamations. The rising intonation signals that an answer to the question is expected, whereas the falling intonation expresses emphasis. Correct application of prosodic elements in language is crucial for effective communication. Sentence stress enables the speaker to give prominence to selected items in a sentence, whereas the intonation pattern marks (among other things) the illocutionary force of a sentence (e.g. declarative, interrogative, etc.), which may not be indicated syntactically. It has to be borne in mind that intonation patterns are language-specific. In English pitch modulation is often used for emphasis or markedness. The language sees both rising and falling intonation, but, unlike in Polish, the latter is also used in questions, both general and specific, next to statements. We do find rising intonation in interrogative sentences, similar to Polish, in the following marked questions: (5) Fighting them? Jack has smashed it? Hasn’t he dropped it all? Such contexts, albeit limited, show we might still find similar intonation patterns in both English and Polish interrogatives (cf. Jassem 1976, Cruttenden [1962] 2014, etc.). In Spanish, the intonation in questions and exclamations is of particular importance. Testing for such intonation is therefore an important diagnostic clue. Not without significance is the interrogative structure, which starts with an appropriate stressed interrogative pronoun. The onset of a question is also marked in spelling by an inverted question mark, next to the regular question mark at the end, as in: (6) ¿Qué tiempo hace hoy? [What is the weather like today?]

11 1. Physiological Foundations of Speech (Material to Dysarthria) ¿De dónde eres? [where are you from?] All in all, intonation understood as changing the voice’s pitch involves rising, falling, or fluctuating the pitch within an utterance. It helps differentiate between interrogative, declarative, or imperative utterances or exclamations in a way characteristic of each language. Let us now sum up the role of the speech organs in speech motor production by noting that different sets of organs are responsible for different aspects of speech. The articulatory organs fulfill fundamental functions in speech production: the tongue movement and its position are responsible for producing specific speech sounds (segments). The lip muscle tension influences the quality of bilabial (p, b, m) and labiodental consonants (v and f); the degree of mouth opening, lip rounding, and jaw and tongue positioning (more precisely the muscles of mastication) affect the quality of vowels. The oral/nasal parameter depends on the soft palate, with the uvula closing or opening the passage between the nasopharynx, the nasal cavity, and the oral cavity, which modifies the airstream pathway and selects the resonator. Phonation, in turn, depends on the vocal folds, larynx, and the movement and contraction of the respiratory muscles. Additionally, prosodic elements such as stress and intonation highlight prominent units within a sentence and help recognize its illocutionary force, respectively. 1.4. THE ROLE OF THE BRAIN AND CEREBELLUM IN EXECUTIVE ASPECTS OF SPEECH PRODUCTION The motor activity of the speech organs is initiated and controlled in the motor cortex in both brain hemispheres, specifically in the precentral gyrus and paracentral lobule near the lateral fissure. These cortical locations, corresponding to the facial muscles, lower jaw, tongue, pharynx and larynx, have to be coordinated in volitional voice production. The motor fibers from the relevant cortical locations run through the corticobulbar tract (including both decussating and non-decussating fibers) to the motor nuclei of cranial nerves of the medulla oblongata (nerves IX, X, XII) and the pons (nerves V and VII). Motor fibers leaving these nuclei innervate the skeletal muscles of the articulatory organs. In addition to the neural structures mentioned above, speech production also engages the cerebellum and basal nuclei.

12 Diagnosing Dysarthria in Adults The cerebellum plays an important role in speech production by coordinating the motor functions of the synergistic and antagonistic muscles. Some of the basal nuclei, especially the caudate nucleus, the putamen, the lateral segment of the globus pallidus and subthalamic nucleus (as part of the extrapyramidal system), collaborate with the extrapyramidal system responsible for voice production. In addition, they affect the activity of neurons in the motor nuclei of the cranial nerves. All the above-mentioned structures play a part in speech production and their activity is beyond conscious control. The mechanism of speech production is complicated and even small damage to one of its parts affects the quality of speech in various ways: lesions of parts of the extrapyramidal system can cause increased muscle tension, known as hypertonia; or decreased tension, known as hypotonia. There can also be uncontrolled movements (hyperkinesia) or limited movements and spasticity (hypokinesia). These disorders will be discussed in greater detail below (cf. also Duus 1989; Prusiński 1989).

2. DYSARTHRIA: CHANGES IN THE CLINICAL PICTURE OF SPEECH Dysarthria is a syndrome involving bulbar–phonation–articulatory disorders caused by lesions in the motor system or pathways innervating the speech apparatus, with the speech planning component remaining intact (Darley, Aronson, Brown 1969). Other definitions of dysarthria characterize it as a speech articulatory disorder resulting from impairment in the neurological mechanisms of voice production, modulation, intensity, and resonance (Johns 1985). In more recent accounts, dysarthria is described as a group of motor speech disorders due to focalized or diffuse damage to the central nervous system, the peripheral nervous system, or both (Communication Independence for the Neurologically Impaired CINI-1994). Dysarthric speech disorders result from lesions in specific locations of the speech motor system, which includes cortical regions (programming the movements of the tongue, lower jaw, pharynx and larynx), subcortical executive regions, as well respiratory, phonatory, and articulatory systems (cf. e.g. Minczakiewicz 1990). The motor functions of the speech organs are controlled by the cortex of the precentral gyrus and paracentral lobule in both hemispheres. Motor fibers involved in voice initiation and production run from the cortex in the corticobulbar tract to the motor nuclei of the cranial nerves V, VII, IX, X, and XII. Motor fibers running from these nuclei innervate the skeletal muscles of the articulatory and respiratory organs, i.e. the muscles of mastication, the muscles of the lips, tongue, palate, larynx, and respiratory muscles (Minczakiewicz 1990, Pruszewicz 1992). Physiological actions underlying speech production require strict coordination of a number of organs constituting the peripheral part of the speech production system. The airstream necessary for phonation engages the lungs, bronchial tubes, and trachea; the larynx amplifies the fundamental frequency of the voice; whereas the pharynx, nasal cavity, and oral cavity are responsible for producing speech sounds and the voice timbre. Impairments in each of the above-mentioned physiological processes involved in speech production may result in dysarthria-type disorders. Respirational disorders occur due to h y p o - or h y p e r f u n c t i o n of the respiratory system, such as mononeuropathy, myasthenia, other muscle disorders, and

14 Diagnosing Dysarthria in Adults chest or abdominal tumors. Diminishing the vital capacity of the lungs, bronchial tubes, and trachea lowers the pitch, reduces the volume of various sounds, deprives some sounds of the required tension (e.g. p, b), and shortens speech phrasing. One of the causes for hyperfunction of the respiratory system is damage to the upper motor neuron (e.g. in the case of amyotrophic lateral sclerosis [ALS] or damage to motor nuclei in the medulla), causing spasticity and desynchronization of respiratory muscles, manifested as explosive speech (Pruszewicz 1992). Distorted phonation may result from pathological position of the vocal folds due to damaged nerves, muscles, or neuromuscular junctions, as well as due to tumors, edemas, or inflammatory conditions in the relevant areas. Phonatory hypofunction occurs with increased adduction of the vocal folds, which leads to heightened pitch, stifled and strangled vocalization. Such disorders often accompany pseudobulbar palsy, dystonia, and cerebellum diseases. In phonation disorders of the mixed type, almost all of the above-mentioned signs are attested, which leads to the production of harsh and hoarse sounds. This may be related to structural damages to vocal folds, such as inflammatory conditions, polyps, and tumors (Pruszewicz 1992). Distorted phonation may also stem from structural damage to organs located above the larynx, causing resonance disorders, such as h y p e r - or h y p o n a s a l i t y, which affect articulation. Excessive (open) nasality or hypernasality is associated with impaired functions of the pharyngeal-palatal region or the soft palate and involves uncontrolled airflow through the nasal cavity during speaking. Hyponasality or closed nasality, on the other hand, is caused by the malfunctioning of nostrils or by opening of the pharyngeal-palatal region. In this impairment, acoustic energy passes through the lips, rendering nasal consonants such as m, n, similar in articulation to the non-nasal b, d. Vowels produced in hyponasality are stifled and dull. General articulatory disorders due to neurological diseases lead to speech distortions: omissions, alternations, sound substitutions or additions. Articulatory disorders never occur in isolation. Patients with all types of dysarthria also exhibit prosodic disorders. In h y p e r p r o s o d i c conditions, we observe increased abruptness of speech and height of pitch, similar to Broca aphasia and manic conditions. In h yp o p r o s o d i c disorders, expression, intensity, and pitch variation are significantly diminished, while speech rhythm may be completely lost. Such disorders typically accompany Parkinsonism or damages to the right

15 2. Dysarthria: Changes in the Clinical Picture of Speech hemisphere. Temporary dysprosodic disorders including rhythm, rate (tempo), and expression impairments are sometimes reported to accompany cerebellum-related dysarthria (Kent et al. 1992; Pruszewicz 1992; Yorkston, Strand, Miller 1993). A commonly accepted c l a s s i f i c a t i o n o f d y s a r t h r i a t y p e s that I use in my clinical practice was offered by Darley et al. (e.g. Darley, Aronson & Brown 1969, 1975; Darley, Spriestersbach & Anderson [1963] 1978). The key criterion in their classification is the d om i n a n t s ymp t om of the disorder. Dysarthria etiologies vary, including degenerative diseases, cardiovascular diseases, or toxic diseases and other conditions. Their severity, though, is contingent on the location and range of the speech motor area lesion. Darley et al. distinguish the following types of dysarthria: – flaccid, – spastic, – ataxic, – hypokinetic, – hyperkinetic, – mixed (1969). This classification is also adopted in this study, albeit with a slight modification introduced to reflect the speech condition precisely. By this token, all mixed types of dysarthria will be described by a compound name, with the first part corresponding to the dominant form of dysarthria and the other part(s) corresponding to the signs and symptoms of other form(s) of the disorder, if any. This proposal has practical ramifications, since the speech condition is dynamic, and various dysarthric symptoms reveal at various stages of its course. In this way, if a patient has been accurately diagnosed, it is possible to follow the dynamics and symptoms of the speech disorder’s development by studying the patient’s medical history.8 If left without any modification, the general name “mixed dysarthria” obscures any relevant symptoms and becomes an umbrella term that could be used to cover all less clearly diagnosed cases. The details of such dysarthria classification are discussed in Chapter Four on dysarthria diagnostics, and the new terminology for mixed cases is dealt with in Chapter Five on dysarthria in neurological diseases. Speech produced by a dysarthria-affected patient is characterized by a number of a r t i c u l a t o r y d i s t o r t i o n s which make it difficult to 8 This diagnostically relevant information is part of the Speech Assessment Method proposed in here, cf. below.

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